Immunological evaluation of late complement component-deficient individuals

Platonov, Alexander E.; Белобородов, В Б; Gabrilovitch, Dmitry I.; Khabarova, Violetta V.; Serebrovskaya, L. V.

doi:10.1016/0090-1229(92)90186-r

Cited by 9 publications

(4 citation statements)

References 22 publications

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“…In so far as our previous studies had not revealed any difference between C8b-and C7-deficient individuals in immunological parameters, the frequency and clinical presentation of meningococcal disease, and immune response to vaccination [3,13,14], the data on C8-and C7-deficient patients were combined to yield a single set of data. The patients, their immune state and molecular basis for complement deficiency, the history of their previous episodes of meningococcal disease have previously been described in detail [3,[11][12][13][14]; patients 1-5, 8, 17, 19-20, 22-27, 29-41, and 45 from the general list of LCCD individuals in Russia [3,11] were recruited in this study. Of 29 LCCD patients, nine experienced one meningococcal episode, 10 two episodes, four three episodes, three four episodes, and two five episodes; one individual, no.…”

Section: Lccd Patientsmentioning

confidence: 99%

Meningococcal disease and polymorphism of FcγRIIa (CD32) in late complement component-deficient individuals

Platonov

Kuijper

Vershinina

et al. 1998

Clinical and Experimental Immunology

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Late complement component‐deficient (LCCD) individuals lack plasma bactericidal activity and are highly susceptible to meningococcal disease. Phagocytosis plays a significant role in immune defence against meningococci and involves FcγRIIa (CD32) on leucocytes. Two allotypic forms are currently recognized: FcγRIIa‐R131 and RIIa‐H131. Neutrophils with the IIa‐H/H131 allotype are more effective in phagocytosis than IIa‐R/R131. We studied the distributions of IIa‐R131 and IIa‐H131 allotypes among 29 Russian LCCD patients who had suffered from recurrent episodes of meningococcal disease. The distribution of IIa‐R/R131 to heterozygous IIa‐R/H131 to homozygous IIa‐H/H131 genotypes was 0.14:0.29:0.57 for LCCD patients who developed the first episode of disease before 10 years of age. The distribution was 0.21:0.64:0.14 for patients who experienced meningococcal disease above the age of 10 years (χ2 = 6, P < 0.05, odds ratio for IIa H/H131 versus R/R131 = 8). Meningococcal disease had a ‘grave’ course in 14 of 31 disease episodes in patients with IIa‐R/R131 and IIa‐R/H131 allotypes, in contrast to 1 of 18 episodes in patients with IIa‐H/H131 allotype (χ2 = 7, P < 0.01, odds ratio = 14). We conclude that IIa‐H/H131 individuals appear to have a higher acquired antibody‐mediated phagocytosis‐dependent resistance to meningococcal disease above the age of 10 years. Additionally, effective CD32‐mediated phagocytosis may restrict the severity of meningococcal disease in LCCD patients with IIa‐H/H131 phenotype.

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Section: Lccd Patientsmentioning

confidence: 99%

Meningococcal disease and polymorphism of FcγRIIa (CD32) in late complement component-deficient individuals

Platonov

Kuijper

Vershinina

et al. 1998

Clinical and Experimental Immunology

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“…Elsewhere we have described Russian LCCD patients, their immune states, infectious morbidity, and meningococcal vaccinations [6, 15, 18]. Briefly, since 1990 31 LCCD patients, 21 males and 10 females, were immunized with meningococcal A + C + W135 + Y vaccine at least 3 months after the last episode of the disease.…”

Section: Methodsmentioning

confidence: 99%

Antibody-Dependent Killing of Meningococci by Human Neutrophils in Serum of Late Complement Component-Deficient Patients

Platonov

Vershinina²,

Käyhty³

et al. 2003

Int Arch Allergy Immunol

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Background: Thirty-one Russian patients with late complement component deficiency (LCCD) who had experienced one to five meningococcal infections were immunized with meningococcal polysaccharide vaccine (A + C + W135 + Y) and were followed for 3–8 years. We investigated the potentially protective killing effect of human neutrophils (PMNL) on serogroup A and W135 meningococci. Methods: Meningococci were incubated in LCCD vaccinee sera in the absence or presence of PMNL, and the number of live bacteria (CFU) was determined by plating onto chocolate agar. Results: When meningococci were incubated in the LCCD sera alone, exponential growth of meningococci occurred despite the presence of meningococcal antibodies. After the addition of PMNL, meningococci were inhibited in their growth or even eliminated. Group A or W135 meningococci were killed effectively by PMNL in 80% of the sera which were collected 1 month to 1 year after vaccination compared to only 40% in the prevaccination LCCD sera (p < 0.05). Three years after vaccination 67% of the LCCD sera were still capable of promoting killing (and even 90% after revaccination). The rate of killing correlated with the concentration of serogroup-specific immunoglobulins. In 83% of the 72 LCCD sera with more than 5 µg/ml anti-group A immunoglobulins the killing of group A meningococci was promoted. By contrast, only 21% of 19 samples with lower specific antibody levels showed a PMNL-mediated meningococcal killing (p < 0.05). The same effect was observed for group W135 meningococci. Conclusion: PMNL kill meningococci during incubation in LCCD serum; this effect increases after vaccination and depends on both specific antibody and complement. Protection by vaccination may therefore be caused by an increased killing capacity of PMNL.

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“…Functional assays for total (CH50) and C7-specific (C7-50) haemolytic complement activity were performed according to standard protocols as detailed elsewhere. 16 Quantification of C7 was carried out using an enzyme-linked immunosorbent assay (ELISA) based on a polyclonal anti-C7 antibody (ELISA P). 17 C7 isoelectric focusing (IEF) typing was performed by haemolytic overlay.…”

Section: Immunological Investigationsmentioning

confidence: 99%

“…16 Quantification of C7 was carried out using an enzyme-linked immunosorbent assay (ELISA) based on a polyclonal anti-C7 antibody (ELISA P).…”

Section: Immunological Investigationsmentioning

confidence: 99%

How partial C7 deficiency with chronic and recurrent bacterial infections can mimic total C7 deficiency: temporary restoration of host C7 levels following plasma transfusion

et al. 1996

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SUMMARYAn apparently completely complement C7-deficient patient with refractory otitis media and two episodes of meningococcal disease was given therapeutic plasma transfusions in 1992 and 1994. Following these transfusions unexpected changes were found in C7 levels. Immediately after transfusion the serum C7 levels failed to rise to the expected levels but then rose to 5-10% of the normal mean during the next 5 days and remained at that level for more than 2 weeks before eventually returning to zero. The patient's DNA genotyped C7 M, and therefore C7 N donor plasma was selected for the second transfusion to allow identification of the source of the C7 circulating post-transfusion. This C7 phenotyped C7 M, demonstrating it to be of recipient origin. Therefore, the apparently completely C7-deficient patient was able to secrete some C7. By a combination of DNA typing and isoelectric focusing of the C7 appearing after transfusion, it was demonstrated that the patient was heterozygous for combined subtotal C6/C7 deficiency (inherited from his father) and a different, so far uncharacterized, subtotal C7 deficiency (inherited from his mother). The low amount of C7 secreted appeared to be constantly consumed, probably by generation of C5b6 as a result of his chronic infection. He had been shown to have circulating C5b6 most of the time, and thus only when sufficient exogenous C7 was given to consume the free C5b6 did his own C7 appear in circulation.

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Immunological evaluation of late complement component-deficient individuals

Cited by 9 publications

References 22 publications

Meningococcal disease and polymorphism of FcγRIIa (CD32) in late complement component-deficient individuals

Meningococcal disease and polymorphism of FcγRIIa (CD32) in late complement component-deficient individuals

Antibody-Dependent Killing of Meningococci by Human Neutrophils in Serum of Late Complement Component-Deficient Patients

How partial C7 deficiency with chronic and recurrent bacterial infections can mimic total C7 deficiency: temporary restoration of host C7 levels following plasma transfusion

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